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Intravascular Crystal Deposition: An Early Clue to the Diagnosis of Type 1 Cryoglobulinemic Vasculitis

机译:血管内晶体沉积:1型冰球蛋白性血管炎的早期诊断线索。

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摘要

Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis.
机译:皮肤小血管血管炎(CSVV)是一种具有广泛鉴别诊断的非特异性发现。将CSVV的皮肤局限性表现与以CSVV作为初始表现的严重威胁生命的系统性血管炎相区分至关重要。确定哪些存在CSVV的患者有全身性疾病的风险可能具有挑战性。通常需要标准的组织病理学评估,直接免疫荧光和血清学评估以排除系统性血管炎。 CSVV可能很少出现1型球蛋白血症。在此,我们报告了在隐匿性多发性骨髓瘤的情况下发生1型冰球蛋白血症的情况。注意到具有明显的血管内晶体形成的CSVV。 CSVV背景下血管内晶体的存在可能代表了诊断1型冰球蛋白血症性血管炎的重要早期线索。

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